Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells.

Assessment of p.Phe508del-CFTR functional restoration in pediatric primary cystic fibrosis airway epithelial cells.

Mutations in the cystic fibrosis transmembrane regulator (CFTR) gene can reduce function of the CFTR ion channel activity and impair cellular chloride secretion. The gold standard method to assess CFTR function of ion transport using the Ussing chamber requires a high number of airway epithelial cel...

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Bibliographic Details
Main Authors: Erika N Sutanto, Amelia Scaffidi, Luke W Garratt, Kevin Looi, Clara J Foo, Michela A Tessari, Richard A Janssen, David F Fischer, Stephen M Stick, Anthony Kicic, AREST CF
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2018-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC5779693?pdf=render

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